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Topiramate in the treatment of severe myoclonic epilepsy in infancy
M Nieto-Barrera1, R Candau, M Nieto-Jimenez
1Paediatric Neurology Unit, Hospital Infantil Universitario Virgen del Rocio, Sevilla, Spain.
Insights
Topiramate (TPM) effectively reduces seizures in severe myoclonic epilepsy in infancy (SME). Many patients experienced significant seizure reduction, with some becoming seizure-free, though side effects were noted with rapid titration.
Area of Science:
- Pediatric Neurology
- Epileptology
- Pharmacology
Background:
- Severe myoclonic epilepsy in infancy (SME) is a rare and severe form of epilepsy.
- Current treatment options for SME have limitations, necessitating exploration of adjunctive therapies.
Purpose of the Study:
- To evaluate the efficacy of topiramate (TPM) as an add-on treatment for reducing seizure frequency in patients with SME.
- To assess the safety and tolerability of TPM in this patient population.
Main Methods:
- A cohort of 18 patients diagnosed with SME was evaluated.
- Topiramate was administered as an add-on therapy, with doses titrated gradually.
- Seizure frequency, type, and adverse events were systematically recorded over a mean observation period of 10.5 months.
Main Results:
- Three patients (16.6%) achieved complete seizure freedom.
- Ten patients (55.6%) experienced a greater than 50% reduction in seizure frequency.
- Nine patients reported side effects, predominantly mild and transient, with a higher incidence in those with weekly dose titration.
Conclusions:
- Topiramate demonstrates significant effectiveness as an adjunctive therapy for managing severe myoclonic epilepsy in infancy.
- Gradual dose titration is recommended to minimize adverse events, which are generally mild and transient.
- TPM offers a promising therapeutic option for improving seizure control in SME patients.
Abstract:
The aim of this study was to assess the effectiveness of topiramate (TPM) as an add-on regimen in reducing seizure rate in a population sample of patients diagnosed with severe myoclonic epilepsy in infancy (SME). Eighteen patients were evaluated. The mean observation time was 10.5 months (range, 6-18 months). Seizure frequency and type were recorded. Topiramate was administered as an add-on regimen at a starting dose of 1 mg kg(-1)and titrated to a maximum of 6-8 mg per kg per day. Different escalation rates were used, mainly weekly or fortnightly increments of dose. Three patients (16.6%) became seizure free, and 10 (55.6%) had a >50% reduction in seizure frequency: six of them (22.2%) achieved a reduction greater than 75%. Side-effects were observed in nine patients, eight with a weekly titration schedule and one with a fortnightly schedule. TPM is effective as adjunctive therapy for SME. Side-effects were mild and transient, generally related to rapid dosage titration.
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